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Child's Nervous System
Published in: Child's Nervous System 12/2008

01-12-2008 | Case Report

Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy (ETV)

Authors: Jothy Kandasamy, Rachel Kneen, Melissa Gladstone, William Newman, Tawil Mohamed, Conor Mallucci

Published in: Child's Nervous System | Issue 12/2008

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Abstract

Introduction

In a small subset of patients, a Chiari malformation can present with signs of raised intracranial pressure due to obstruction of cerebrospinal fluid flow or with the raised intracranial pressure as the primary pathological driving force resulting in tonsillar herniation.

Case report

The authors report a unique case in a 14-year-old boy with a Chiari malformation type 1–syringomyelia complex with slit-like ventricles. We have successfully managed the acute presentation of raised intracranial pressure, mimicking idiopathic intracranial hypertension, utilizing a frameless stereotactic image-navigated endoscopic third ventriculostomy alone. We present the preoperative and postoperative radiological and ophthalmological findings and discuss the possible mechanisms related to the pathophysiology and treatment in this case. The patient’s symptoms resolved immediately postoperatively with resolution of papilledema. At 18-month follow-up, the patient remains entirely asymptomatic and the need for craniovertebral decompression appears to have been obviated.

Conclusion

We advocate that in the Chiari malformation type 1–syringomyelia complex with normal or small ventricles, patients presenting with isolated signs and symptoms of raised intracranial pressure alone can be safely and effectively managed with an electromagnetic-guided stereotactic endoscopic third ventriculostomy as a primary treatment option.
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Metadata
Title
Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy (ETV)
Authors
Jothy Kandasamy
Rachel Kneen
Melissa Gladstone
William Newman
Tawil Mohamed
Conor Mallucci
Publication date
01-12-2008
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 12/2008
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-008-0672-y

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